Systemic Skeletal Diseases

  • Simone Waldt
  • Dirk Müller
  • Thomas Link
Chapter

Abstract

Osteopoikilosis is a rare and benign disease characterised by multiple round osteosclerotic lesions throughout the whole skeletal system.

Osteopoikilosis is asymptomatic and is usually found incidentally. In ca. 10–25% of cases, the disease is associated with various skin lesions. An autosomal dominant inheritance can be assumed based on family examinations. Osteosclerotic lesions show histological similarities to bone islands and osteoma (Chap. 36).

The radiographic appearance is pathognomonic: mostly periarticular, meta- and epiphyseal, multiple, round, or ovoid foci of sclerosis (Fig. 38.1). Predilection sites are the long tubular bones. Occurrences in the carpal and tarsal bones, pelvis, and scapula are also common. Although most lesions are constant over the course of time, they can also be dynamic, which means increasing or decreasing in size and number. As a rule lesions exhibit no increased activity on scintigraphy.

Differential diagnosis considerations include osteoblastic metastases, mastocytosis, and tuberous sclerosis. Usually the symmetric distribution, the periarticular location, and the typical morphology of lesions as well as the missing clinical symptoms allow for the correct diagnosis. Therefore, in most cases apart from conventional radiographs no additional imaging modalities are necessary. In doubtful cases particularly a normal bone scan may support the diagnosis.

Synonym: Voorhoeve disease.

Keywords

Adenoma Bismuth Glaucoma Hypothyroidism Ceramide 

Bibliography

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Simone Waldt
    • 1
  • Dirk Müller
    • 2
  • Thomas Link
    • 3
  1. 1.Diagnostische RadiologieKlinik für Radiologie und Neuroradiologie, Alfried Krupp KrankenhausEssenGermany
  2. 2.Institut für RadiologieKlinikum rechts der Isar der Technischen Universität MünchenMünchenGermany
  3. 3.Department of RadiologyUniversity of Southern CaliforniaLos Angeles, CAUSA

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